Connective tissue diseases and vasculitis Flashcards
(44 cards)
What are connective tissue diseases
Autoimmune rheumatic diseases
What is Systemic Lupus Erythematosus?
An inflammatory multisystem autoimmune disorder.
Who would most commonly present with SLE?
A female Afro-Caribbean of 20-40yrs
identify some factors related to the aetiology of SLE
genetics
oestrogen levels
immunological factors
environmental- UV light, smoking, Epstein-Barr Virus
Describe the pathogenesis of SLE
After apoptosis of cells cellular remnants remain called blebs which carry self antigens on their surface. These are normally cleared up but instead they travel to lymphoid tissue where they sensitise T-cells. This leads to the production of autoantibodies by B-cells, triggering of the complement system and abnormal cytokine production.
What characterises the pathology of SLE?
Deposition of IgG antibodies and complement proteins and influx of neutrophils + lymphocytes. Also vasculitis
There are many variant clinical manifestations of SLE. Joint involvement is the most common manifestation. What features may be shown
synovitis and joint tenderness 2+ joints
myalgia
Jaccoud’s arthropathy
How does SLE manifest in the skin?
characteristic butterfly rash erythema on face photosensitivity purpura urticaria alopecia Raynaud's phenomenon
As well as resulting in symptoms related to the joints and skin, what other ways may SLE present?
interstitial lung disease, pleural effusions pericarditis, endocarditis lupus nephritis depression, delirium, Psychosis, Sjorgen's syndrome
Main investigations for SLE?
blood test- FBC- leucopenia/thrombocytopenia/lymphopenia
autoantibodies- ANA, anti-dsDNA, anti-Ro, anti-Sm, anti-La
complements C3 and C4
treatment of SLE?
avoid excessive sun exposure
NSAIDs
hydroxychloroquine
corticosteroids
What is Primary Sjorgen’s syndrome? What are the main symptoms of Sjorgen’s?
The syndrome of dry eyes in the absence of RA and other autoimmune diseases
dry eyes, mouth, skin, vagina
fatigue
salivary and parotid gland enlargement
Which auto-antibodies are raised in Sjorgen’s?
anti-Ro, anti-La
Sjorgen’s increases risk of what serious condition?
Lymphoma
What are the two major characteristics of the pathology of Systemic Scleroderma (SSc)
Vasculopathy- widespread vascular damage
fibrosis- over lower dermis and internal organs (treatment symptom based or organ specific)
What is the main red flag feature of Systemic scleroderma?
Raynaud’s syndrome
What is the difference between LcSSc and DcSSc?
Limited and Diffuse cutaneous scleroderma differ in frequency 70%:30%, distribution (limited is restricted to hands, feet, face and forearms), and the clinical feature shown.
What are the main features of SSc?
Raynaud's Thickened skin- distribution varies- diffuse/limited? Tight skin other face, small mouth and beaky nose Telangiectasia GI trouble pulmonary hypertension pulmonary fibrosis myocardial fibrosis scleroderma renal crisis
Which are the two main autoantibodies shown in Scleroderma?
anti-centromeres
anti-topoisomerase
How is Raynaud’s syndrome treated?
hand warmers
vasodilators- Calcium channel blockers e.g. nifedipine
PDE-5 inhibitors
prostacyclins
What is the characteristic symptoms of the following conditions? Inflammatory myopathies, polymyalgia rheumatica and fibromyalgia
Myopathy- proximal muscle weakness
polymyalgia rheumatica- pain and stiffness
fibromyalgia- pain and fatigue
The aetiology of inflammatory myopathies are _________. they are more common in _________. they have an associated risk of ___________. When the skin is involved it is called ___________.
unknown
women
malignancy
dermatomyositis
What are the main clinical features of myalgia?
proximal muscle weakness
myalgia
difficulty with specific actions
insidious, over months or acute onset.
What three signs distinguish polymyositis from dermatomyositis?
Gottron’s sign (purple discolouration of knuckles)
shawl sign
heliotrope rash (purple discolouration around eyes)
